RÉSUMÉ
El neuroblastoma congénito es el tumor sólido maligno más frecuente en el período neonatal. La forma de presentación suele ser por diagnóstico prenatal o por una masa abdominal. Su estadificación permite clasificarlo en grupos de riesgo con pronóstico y tratamiento diferentes. En el período neonatal, se caracteriza por la alta tasa de regresión espontánea y el buen pronóstico (supervivencia libre de enfermedad a los 5 años superior al 90 %). Se presenta un caso clínico de neuroblastoma congénito cuya forma de presentación, shock e hipertensión, solo estaba descrita en otra ocasión antes. El tratamiento antihipertensivo, junto con la quimioterapia sistémica, produjo el control clínico y la mejoría del paciente.
Congenital neuroblastoma is the most frequent malignant solid tumor in the neonatal period. The clinical presentation is usually either by prenatal diagnosis or by palpation of an abdominal mass. Staging allows classifying it according to risk groups with a different prognosis and treatment. In the neonatal period, it is characterized by a high rate of spontaneous regression and good prognosis (disease-free survival at 5 years greater than 90 %). We present a clinical case of congenital neuroblastoma whose presentation, shock and hypertension, was only described on a previous occasion. Antihypertensive treatment along with systemic chemotherapy produced clinical control and patient improvement.
Sujet(s)
Humains , Mâle , Nouveau-né , Choc , Hypertension artérielle , Néonatologie , NeuroblastomeRÉSUMÉ
Los neuroblastomas congénitos cervicales son muy raros. Esta forma de cáncer infantil se forma en el tejido nervioso y por lo general suele presentarse con mayor frecuencia en las glándulas suprarrenales. Aunque puede aparecer prenatalmente, es más frecuente que se diagnostique en el primer año de vida. Son tumores agresivos con una alta mortalidad. En casi todos los casos (50-60 por ciento de los mismos), para cuando se detecta un neuroblastoma, ya se ha diseminado a otras partes del cuerpo. Se presenta un caso de recién nacido que las primeras 24 horas, muestra una historia de compromiso de vías respiratorias y digestivas asociado a una masa cervical sólida y parálisis de XII par craneal.
Cervical congenital neuroblastomes are very rare. This form of infantile cancer forms in the nervous tissue and generally it uses to appear more frequently in the suprarenal glands. Although they may appear prenatally, they are more frequent in the first year after birth. They are aggressive tumors with a high mortality. In almost all the cases (50/60 percent of them), when a neuroblastome is detected, it is already disseminated to other parts of the body. We present the case of a newborn who shows a history of respiratory and digestive tracts compromise associated to a solid cervical mass and XII cranial par paralysis during the first 24 hours after birth.
Sujet(s)
Humains , Femelle , Nouveau-né , Tumeurs de la tête et du cou , Neuroblastome/chirurgie , Neuroblastome/diagnostic , Présentations de casRÉSUMÉ
A two-month-old female child presented with discrete skin-colored papules on the forehead and scalp. She was earlier diagnosed to have neuroblastoma of the adrenal gland which had metastasized to the liver. Histological sections of the skin lesion showed a nodule composed of sheets of cells with abundant eosinophilic cytoplasm and an occasional grooving of the nucleus. These cells were CD 68 positive. Eosinophilic abscesses were seen in between the neoplastic cells.
RÉSUMÉ
Neuroblastoma is the most common intraabdominal malignant tumor of childhood, with 40% arising from the adrenal gland. Bilateral adrenal involvement from synchronous development or metastatic spread of tumor is rarely seen in children with neuroblastoma. The patient was born with a spontaneous vaginal delivery. Birth weight was 3,200 g. Fetal ultrasonography showed a left adrenal cystic mass. At two weeks of age, she was admitted due to a massive abdominal distension and tachypnea. Percutaneous ultrasonography guided biopsy of the left adrenal mass was performed. The result of the biopsy was neuroblastoma. Vincristine and cyclophosphamide were administerd intravenously and 450 cGy of irradiation was added. Left adrenalectomy was accomplished and postoperative course was uneventful. The patient received cancer chemotherapy with a combination of carboplatin, ifosfamide and VP-16 and is now being followed up for three months. We have experienced a case of congenital bilateral neuroblastoma and report the case with brief review of related literatures.
Sujet(s)
Enfant , Humains , Glandes surrénales , Surrénalectomie , Biopsie , Poids de naissance , Carboplatine , Cyclophosphamide , Traitement médicamenteux , Étoposide , Ifosfamide , Neuroblastome , Tachypnée , Échographie , Échographie prénatale , VincristineRÉSUMÉ
Neoplasms presenting at birth or within the first month of life are defined as congenital tumors. The principal components of this congenital tumors are neuroblastoma, leukemia, brain tumors and sarcomas. The neuroblastoma is the most common accounting for 15~50% of all tumors in this group. It most often presents with an abdominal mass due to adrenal-retroperitoneal primary or hepatomegaly resulting from extensive metastasis. Most often the primary site is adrenal but other loci include the retroperitoneum, mediastinum, pelvis, etc. This 2-day-old female presented with hepatomegaly and a left adrenal mass at birth, first detected by ultrasonography. On the first day, she suffered from hematemesis and bradycardia. She died on the second day. Postmortem examination revealed massive metastatic tumor nodules in the liver and a well-demarcated round mass, 4 cm, in the left adrenal, with necrosis and hemorrhage. Microscopic findings revealed largely undifferentiated neuroblastoma with focal neuronal differentiation and areas of necrosis and calcification in the background of fine fibrovascular stroma. Other metastatic foci were detected in the right adrenal and pituitary gland.